Clinical manifestations and advances in hereditary research of alternating hemiplegia of childhood

Alternating hemiplegia of childhood(AHC) is a hereditary disease characterized by hemiplegia spells,abnormal eye movements,dystonia and cognitive impairment.There are three phases of the disease.Each phase has its unique clinical symptoms:phase 1-abnormal eye movements and dystonia;phase 2-hemiplegia spells; phase 3-permanent cognitive impairment.The severity of cognitive impairment depends on the time of onset of hemiplegia spells:the earlier the onset is,the worse outcome will be.No effective treatment has been established.Thetreatments currently include:avoiding predisposing factors and taking drugs such as flunarizine to prevent hemiplegia attacks,in the inter-ictal stage;and sedation during hemiplegia attacks.According to the latest research,AHC is caused by the de novo mutation of gene ATP1A3. Key words: Alternating hemiplegia of childhood;  ATP1 A3 ;  Abnormal ocular movements;  Dystonia